[Multiple bladder diverticula caused by occipital horn syndrome]. / Diverticulose vésicale liée à un syndrome de la corne occipitale.

We report on the case of a child who presented with recurrent, multiple, and voluminous bladder diverticula. Bladder diverticula are defined as a herniation of the mucosa through the bladder muscle or the detrusor. Causes are numerous and diverticula can be classified into primary congenital diverticula (para-ureteral - or Hutch diverticula - and posterolateral diverticula); secondary diverticula (resulting from chronic mechanical obstruction or from neurological disease; and diverticula secondary to connective tissue or muscle fragility. The latter is seen in disease entities such as prune belly syndrome, Ehlers-Danlos syndrome, cutis laxa syndrome, OHS (occipital horn syndrome), Menkes disease, and Williams-Beuren syndrome. In this patient, the cause of these diverticula was OHS, a genetic, recessive X-chromosome-linked syndrome, responsible for abnormal tissue caused by a disorder in copper metabolism. This case reminds us of the importance of pushing the diagnostic workup when presented with multiple and/or large bladder diverticula, and in particular to search for rare malformation syndromes after exclusion of an obstacle.

Long-Term Functional, Cosmetic and Sexual Outcomes of Hypospadias Correction Performed in Childhood.

OBJECTIVE: Hypospadias surgery, especially when performed early in life, may have a significant impact on the urinary and sexual functions in an adult. Because the literature is still limited, this paper assesses long-term functional, cosmetic and sexual results of hypospadias repair performed in childhood. PATIENTS AND METHODS: The study includes 275 patients older than 12 years treated for a hypospadias by an Onlay, Mathieu, Duplay, or Duckett's technique between January 1990 and December 2000. Flowmetry results were retrospectively obtained from patients' charts. The Paediatric Penile Perception Score (PPPS), the Hypospadias Objective Scoring Evaluation (HOSE) and the IIEF-5 score (when older than 16 years old) questionnaires were used to assess cosmetic and sexual results. The PPPS is designed to assess both penile self-perception with regard to meatus, glans, skin and general appearance. The HOSE is a five-point scoring system designed to allow an objective appraisal of the outcome of hypospadias repair, based on evaluating meatal location, meatal shape, urinary stream, straightness of erection, and the presence and complexity of any complicating urethral fistula. RESULTS: Qmax were within age-adjusted references, independent of the surgical technique, with median (range) Qmax of 18.8 ml/s (range 3-45, n = 136). Patients expressed a high satisfaction for every single item of the penile perception scale (PPPS), with mean values between 2 (satisfied) and 3 (very satisfied). Eighty-two percent were satisfied or very satisfied of the overall evaluation of penile appearance. Eighty-one percent of patients had a normal erectile function (IIEF-5 >22; n = 35/43). CONCLUSIONS: Taking into account the limitation of a small number of patients resulting from a low 21% questionnaire's response rate, the results of this study align with previous reports from the literature and confirms that hypospadias repair using standard techniques results in acceptable functional, cosmetic and sexual outcomes. This study highlights the need of developing a set of standard approved outcomes assessments tools for evaluating the long-term impact of hypospadias repair performed in infancy.

Secondary retroperitoneal teratoma.

Retroperitonal teratomas are rare. We report on a case of a retroperitoneal secondary localisation of a gonadal teratoma in a patient who had developed primary testicular teratoma 12 years previously. The retroperitoneal mass was detected with an abdominal CT requested for the management of a non-specific abdominal pain. CT and MRI examinations showed cystic retroperitoneal masses combined with calcifications and peripheral enhancement. Review of the literature is presented, including the common differential diagnoses to be considered.

Does site-specific labelling and individual processing of sextant biopsies improve the accuracy of prostate biopsy in predicting pathological stage in patients with T1c prostate cancer?

OBJECTIVE: To evaluate whether individual labelling and processing of the sextant of origin improves the accuracy of prostate biopsy in predicting the final pathological stage after radical prostatectomy in patients with T1c prostate cancer. PATIENTS AND METHODS: The charts of 386 patients treated for prostate cancer by radical prostatectomy between January 1996 and June 1999 were reviewed. In all, 124 patients fulfilled the following inclusion criteria: no abnormality on digital rectal examination (DRE) or transrectal ultrasonography, a prostate specific antigen (PSA) level before biopsy of < or = 20 ng/mL, and prostate cancer diagnosed after one set of random sextant biopsies, with the cores being submitted in six separate containers individually labelled for the sextant of origin. RESULTS: Within this series of patients with a low tumour burden, the preoperative PSA, biopsy Gleason score and unilateral vs bilateral involvement were not significant predictors of disease extension. The percentage of positive cores and the number and topography of positive sextants were both statistically significant predictors of organ-confined disease. Although these two variables appeared to be statistically equivalent on a first analysis in the overall series, a subgroup of patients was identified who benefited from the complete topographical information, i.e. those 52 (42%) patients with a Gleason score of < 7, 25-75% positive biopsies and < or =3 positive sextants. CONCLUSION: These results support the individual labelling of biopsy cores in selected patients with a normal DRE and a moderately elevated PSA, as it helps to better predict the final pathological stage. This substantial benefit outweighs the additional effort by the pathologist.

Assessing the risk of unsuspected prostate cancer in patients with benign prostatic hypertrophy: a 13-year retrospective study of the incidence and natural history of T1a-T1b prostate cancers.

OBJECTIVE: To determine the incidence and natural history of stage T1a-T1b prostate cancer in patients undergoing surgery for benign prostatic hypertrophy (BPH), and thus evaluate the effect that recent medical and 'minimally invasive' treatments (which provide no prostate sample for pathological examination) might have on the percentage of patients with unsuspected prostate cancer. PATIENTS AND METHODS: A series of 1648 patients undergoing surgery for BPH over a 13-year period were reviewed retrospectively; the period overlapped the introduction of serum prostate specific antigen (PSA) as a detection method. RESULTS: Stage T1 prostate cancer was found in 182 patients (11%), comprising 126 (11%) of 1199 transurethral resections and 56 (12%) of 449 open enucleations. The introduction of systematic PSA assays gradually reduced the mean incidence of T1 cancer from 23% to 7%, with a greater effect on T1b (from 15% to 2%), while the incidence of T1a remained nearly constant (+/-5%). The pathological features of surgical specimens from 43 radical prostatectomies undertaken for T1 tumours were reviewed. Locally advanced disease (stage >/=pT3) was apparent in 13% of T1a and 28% of T1b tumours. Amongst the patients electing for surveillance, only 8% of those with T1a progressed within 30-97 months of follow-up (mean progression time 73 months), whereas 29% of those with stage T1b progressed within 36 months of follow-up (mean progression time 17 months). CONCLUSION: These results show that the use of the PSA assay has decreased but not suppressed the incidence of pT1 prostate cancer, with a greater effect on those tumours at higher risk of progression (T1b). This suggests that the detection of prostate cancer based on PSA and transrectal ultrasonography is appropriate for screening patients and is sufficiently accurate that treatments for BPH that provide no pathological materials can be applied safely.

Urothelial lesions in Chinese-herb nephropathy.

Rapidly progressive renal fibrosis after a slimming regimen including Chinese herbs containing aristolochic acid (AA) has been identified as Chinese-herb nephropathy (CHN). We reported urothelial atypia in three patients with CHN, with the subsequent development in one patient of overt transitional cell carcinoma (TCC). Therefore, it was decided to remove the native kidneys, as well as the ureters, in all patients with CHN. Nineteen kidneys and ureters removed during and/or after renal transplantation from 10 patients were studied to assess critically urothelial lesions and to characterize the cellular expression of p53, a tumor-suppressor gene overexpressed in several types of malignancies. Multifocal high-grade flat TCC in situ (carcinoma in situ; CiS) was observed, mainly in the upper urinary tract, in four patients, a prevalence of 40%. In one of those patients, a superficially invasive flat TCC of the right upper ureter, as well as two additional foci of noninvasive papillary TCC, were found in the right pelvis and left lower ureter, respectively. This patient also presented recurrent noninvasive papillary TCC of the bladder. Furthermore, in all cases, multifocal, overall moderate atypia was found in the medullary collecting ducts, pelvis, and ureter. All CiS and papillary TCC, as well as urothelial atypia, overexpressed p53. These results show that the intake of Chinese herbs containing AA has a dramatic carcinogenic effect. Carcinogenesis is associated with the overexpression of p53, which suggests a role for a p53 gene mutation. The relationship of this mutation with the reported presence of AA DNA adducts in the kidney remains to be explored.

Urachal neuroblastoma: first case report.

Tumours of the urachus are exceptional in children. They represent 0.01% of all tumours and consist of mucosecretory adenocarcinoma and, more rarely, transitional cell carcinoma. We report a 6-month-old child with a urachal mass which, following biopsy, was shown to be a neuroblastoma.

Testicular torsion after previous orchidopexy for undescended testis.

We report one case of acute testicular torsion following orchidopexy for an undescended testis. A review of the literature reveals only ten similar cases. History of a previous testicular surgery should not preclude the possibility of a torsion in that testicle. We conclude that at orchidopexy for an undescended testis, eversion of the tunica vaginalis is an essential step to avoid any future torsion.

Urodynamic and electrophysiological testing in pediatric neurourology.

In our institution, children with voiding disorders are submitted to a detailed evaluation comprising a careful clinical examination, laboratory tests, imaging (kidney ultrasonography and voiding cystourethrography) and urodynamic tests. Depending on the etiology of voiding dysfunction, children are subjected to a non invasive or an invasive urodynamic evaluation. Non invasive urodynamic testing comprises a free urinary flowmetry associated or not with an EMG recording of the pelvic floor. Invasive urodynamic testing consists of a pressure-flow study or a video-XR-urodynamic test. In rare instances, genitourinary electrophysiological investigations will be performed. Herein we describe these different urodynamic and electrophysiologic tests.

Is adrenalectomy part of radical nephrectomy?

Standard radical nephrectomy entails en bloc removal of the kidney together with Gerota's fascia and the ipsilateral adrenal. Thanks to the refinement of imaging techniques (ultrasound, CT and MRI), smaller tumors are being diagnosed. In addition, direct extension to the adrenal gland or adrenal metastasis can be detected in most cases. This is why several authors reserve adrenalectomy for large and/or upper pole tumors or abnormal appearing glands on preoperative CT-scan. However, preoperative diagnosis is not always accurate. Furthermore, micrometastatic adrenal invasion at the time of nephrectomy and late recurrences in the persistent adrenal have been documented, so that partisans of adrenalectomy only spare the adrenal in exceptional cases. The authors have reviewed several series in the litterature as well as there own, and conclude that ipsilateral adrenalectomy can be omitted for small middle- or lower pole tumors when the adrenal appears normal on CT and during the surgical intervention.

[Complete radical prostatectomy and positive lymph nodes (stages pT1 to 4, N1 to 3, M0, D1)]. / Prostatectomie radicale totale et ganglions positifs (stades pT1 à 4, N1 à 3, M0, D1).

Lymph node metastases are rarely detected during radical prostatectomy (55/647 patients in our series or 8.5%) and several authors consider that lymphadenectomy is unnecessary in most cases. Criteria based on clinical stage, PSA and tumor grade have been elaborated in order to avoid pelvic lymph node dissection in a low risk population. It is commonly admitted that patients with clinically localized prostate cancer, a PSA level < 10 ng/ml, and a Gleason score < 7 could be spared a pelvic lymphadenectomy. In our series, these patients account for 12% of positive nodes. The best treatment for prostate cancer patients with a nodal disease is controversial. We compare the evolution of two groups of patients: radical prostatectomy alone or combined with an immediate adjuvant hormonal treatment. We observe a difference between the two groups for biological progression (PSA failure) but not yet for clinical progression nor for survival as our mean follow-up in only 6 years.

[Correction of severe median hypospadias. Review of 77 cases treated by the onlay island flap technic]. / Correction des hypospades de gravité moyenne. Revue de 77 cas traités par la technique du "onlay island flap".

Seventy-seven hypospadias, selected from a series of 504 treated in our institution, were operated using the pediculated transverse "onlay" preputial graft. We try to demonstrate that this technique produces excellent functional and cosmetic results. Chordee can be corrected using extensive dissection of the urethral plate with association, if necessary, of a Nesbit type of dorsal plication. Complications rate, in our hands, is lower in comparison to the technique described by John Duckett, where the urethral plate is divided and a complete urethral tube is performed. We report 4 fistulas, one distal end breakdown of the repair and one postoperative hematoma that had to be revised. Because of this low complication rate, in many cases, the "onlay" pediculated graft replaces advantageously the Duckett type of repair.

Adjuvant radiation therapy does not cause urinary incontinence after radical prostatectomy: results of a prospective randomized study.

PURPOSE: We analyzed the potential influence of adjuvant radiotherapy on urinary continence after radical prostatectomy. MATERIALS AND METHODS: A total of 100 patients with N0M0 prostate cancer randomized in a prospective study on postoperative radiotherapy for locally advanced disease (positive surgical margin, capsular perforation and/or seminal vesicle infiltration) were studied. Objective pad weighing tests corroborated by direct personal interviews were used to evaluate urinary continence at regular postoperative intervals. RESULTS: Of the patients 48 received 60 Gy. external radiotherapy with 18 MV photon beams between 12 and 16 weeks postoperatively, and 52 were followed expectantly. Risk factors were similar in both groups. With a mean followup of 24 months, no difference in complete urinary continence was observed. Of the irradiated group 77% and of the surveillance group 83% were totally dry. The fate of the bladder neck had no significant influence on final continence status, although there was a trend for faster recovery when the bladder neck was preserved. CONCLUSIONS: In this prospective randomized study 60 Gy. external radiation therapy administered between 3 and 4 months after radical prostatectomy for pathologically locally advanced prostate cancer had no significant influence on urinary continence.

Megaureter in childhood: conservative or surgical management?

Antenatal and incidentally diagnosed megaureters can be safely treated conservatively after careful work up by combined imaging technics. Serial and repeated imaging are non the less usually necessary to confirm the diagnosis. This aspect could be fastidious and expensive. In case of symptoms, breakthrough infections or loss of kidney function, surgical treatment should be considered and good results can be expected. Refluxing megaureter has to be considered as high grade reflux and surgical approach is more often suggested. The authors have reviewed the experience of 139 patients with megaureters treated in the last decade to illustrate those facts.

[Severe and unexpected occurrence of water-electrolyte disorders in the postoperative period]. / Désordres hydro-électrolytiques sévères et inattendus survenus en période post-opératoire.

Unexpected severe electrolyte imbalance in the postoperative period. Report two cases. Two cases of inappropriate secretion of antidiuretic hormone are reported. In each patient, the physiopathology is reviewed. Differential diagnosis and treatment of hyponatremia is discussed.

[Recurrence following radical surgery for prostatic cancer. Analysis of clinical, biological and anatomo-pathological prognostic factors]. / Récidive après chirurgie radicale pour cancer prostatique. Analyse des facteurs pronostiques cliniques, biologiques, et anatomopathologiques.

To better characterize risk factors of progression (or recurrence) of prostate cancer after radical surgery, we analysed clinical and biological preoperative characteristics and post-operative pathology results in a series of 179 patients who underwent radical prostatectomy between January 1, 1993 and December 31, 1994. The mean follow-up in the series is 36 months (24-36). 39 patients treated before radical prostatectomy by hormonotherapy or surgery (TURP, TULIP) were excluded from analysis. 28 patients treated with immediate adjuvant therapy were also excluded from the study on risk factors of recurrence. Clinical understaging is 37% (50/134 patients with stage T1-T2 have extracapsular extension or invasion of seminal vesicles). Preoperative PSA value is related to the pathologic stage. Extracapsular disease was found in 17% and 46% when PSA was < 4 ng/ml or > 10 ng/ml respectively, thereby confirming the poor staging value of preoperative PSA alone. Analysis of the surgical margins demonstrates a statistically significant difference (p = 0.018) between patients with a preoperative PSA < 10 ng/ml (22% of positive margins) and those with a PSA > 10 ng/ml (42% of positive margins). Predictive factors of recurrence were analyzed in the 112 patients who have not received pre- or postoperative treatment. The respective impact of clinical stage, preoperative PSA value, Gleason score, invasion of prostatic apex, capsular perforation, surgical margins, invasion of seminal vesicles or of pelvic lymph nodes, and invasion of intraprostatic, intracapsular or extraprostatic nerves were evaluated. In T3 cases, we observe 50% recurrence (but only 4 patients fall into this group) versus 14% in clinically localized tumors (T1c-T2c). No recurrence is detected when preoperative PSA is < 4 ng/ml; on the contrary 21% of patients with a PSA > 10 ng/ml recurred. Infiltration of the apex does not influence prognosis. In our experience, capsular perforation is a worse prognostic factor than positive surgical margins, the respective rate of failure being 25% and 17% respectively. Invasion of extraprostatic nerves increases the risk of failure compared to capsular perforation alone (31% vs 18%). Seminal vesicles invasion significantly worsens prognosis (50% vs 13% recurrence respectively; p = 0.024). All patients with positive lymph nodes recurred (p = 0.001).

The external vacuum device in the management of erectile dysfunction.

The effectiveness of a vacuum erection device was evaluated in a 3-year prospective trial involving 110 men with erectile disorders. The patients ranged from 36 to 75 years of age (average: 61). Fifty-seven had chosen the vacuum device as a first treatment attempt; 27 were on intracavernous self-injections with a vasoactive agent but wanted another treatment; and several treatment modalities had failed in the remaining 26 patients. At the 3-month follow-up, the patients were mailed a questionnaire to evaluate the quality of sexual intercourse with the vacuum constriction device. Partner satisfaction was evaluated in parallel. Improvement in sexual parameters as well as difficulties and side effects induced by the device were investigated. Our results confirm the efficacy of the vacuum constriction device in the treatment of erectile dysfunction in selected patients.

Free to total prostate-specific antigen (PSA) ratio improves the discrimination between prostate cancer and benign prostatic hyperplasia (BPH) in the diagnostic gray zone of 1.8 to 10 ng/mL total PSA.

OBJECTIVES: Improved discrimination between prostate cancer (PC) and benign prostatic hyperplasia (BPH) is clearly needed. Our aim in this study was to evaluate whether the free to total prostate-specific antigen (PSA) ratio would be useful in the gray zone of 1.8-10 ng/mL total PSA range. METHODS: In a consecutive series of 435 clinic patients referred for prostate evaluation, 308 had a total PSA < 10 ng/mL (92 had PC and 216 BPH). Free and total PSA were measured, and the free to total PSA ratio calculated. RESULTS: Total PSA values were significantly different between the two groups. For the 200 patients with a total PSA < 6 ng/mL, no significant difference in total PSA values were seen (P = 0.411), whereas free to total PSA ratios remained statistically different (P < 0.001). Receiver operating characteristic (ROC) curve analysis comparing the performances of total PSA over the ratio of free to total PSA showed a clear advantage for the ratio at all sensitivity levels. CONCLUSIONS: These data demonstrate that in a significant number (n = 308) of prostatic patients in the diagnostic gray zone of 1.8-10 ng/mL total PSA, the routine use of free to total PSA might be advantageous in discriminating between cancer and benign hyperplasia. This advantage remained for total PSA < 4 ng/mL. Further study is warranted to confirm these findings in an unselected population.